Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family Journal Article


Authors: de Alava, E.; Gerald, W. L.
Article Title: Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family
Abstract: Ewing's sarcoma (ES) and primitive neuroectodermetal tumor (PNET) are members of a tumor family consistently associated with chromosomal translocation and functional fusion of the EWS gene to any of several structurally related transcription factor genes. Similar gene fusion events occur in other mesenchymal and hematopoietic tumors and are tumor-specific. The resulting novel transcription factor-like chimeric proteins are believed to contribute to tumor biology by aberrant regulation of gene expression altering critical controls of cell proliferation and differentiation. These tumor-specific molecular rearrangements are useful for primary diagnosis, may provide prognostic information, and present potential therapeutic targets. The recent advances in our understanding of the molecular biology of ES and PNET represent a paradigm for the application of the basic biology of neoplasia to clinical management of patients.
Keywords: human cell; review; cell proliferation; mesenchyme cell; transcription factor; cell differentiation; transcription factors; cell transformation, neoplastic; ewing sarcoma; chimera; gene expression regulation, neoplastic; gene rearrangement; recombinant fusion proteins; gene fusion; chromosome translocation; translocation, genetic; target cell; molecular biology; protein family; sarcoma, ewing's; neuroectoderm tumor; neuroectodermal tumors, primitive; tissue specificity; humans; human; priority journal
Journal Title: Journal of Clinical Oncology
Volume: 18
Issue: 1
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 2000-01-01
Start Page: 204
End Page: 213
Language: English
PUBMED: 10623711
PROVIDER: scopus
DOI/URL:
Notes: Export Date: 18 November 2015 -- Source: Scopus